American Society of Hematology 2020 guidelines for sickle cell disease: prevention, diagnosis, and treatment of cerebrovascular disease in children and adults

MR DeBaun, LC Jordan, AA King, J Schatz… - Blood …, 2020 - ashpublications.org
Background: Central nervous system (CNS) complications are among the most common,
devastating sequelae of sickle cell disease (SCD) occurring throughout the lifespan …

The multifaceted role of ischemia/reperfusion in sickle cell anemia

RP Hebbel, JD Belcher… - The Journal of Clinical …, 2020 - Am Soc Clin Investig
Sickle cell anemia is a unique disease dominated by hemolytic anemia and vaso-occlusive
events. The latter trigger a version of ischemia/reperfusion (I/R) pathobiology that is singular …

Haptoglobin therapeutics and compartmentalization of cell-free hemoglobin toxicity

PW Buehler, DJ Schaer - Trends in molecular medicine, 2020 - cell.com
Hemolysis and accumulation of cell-free hemoglobin (Hb) in the circulation or in confined
tissue compartments such as the subarachnoid space is an important driver of disease …

Blood transfusion for preventing primary and secondary stroke in people with sickle cell disease

LJ Estcourt, R Kohli, S Hopewell… - Cochrane Database …, 2020 - cochranelibrary.com
Background Sickle cell disease is one of the commonest severe monogenic disorders in the
world, due to the inheritance of two abnormal haemoglobin (beta globin) genes. Sickle cell …

Sickle cell disease and stroke

D Hirtz, FJ Kirkham - Pediatric Neurology, 2019 - Elsevier
Cerebral infarction is a common complication of sickle cell disease and may manifest as
overt stroke or cognitive impairment associated with “silent” cerebral infarction on magnetic …

From stress to sick (le) and back again–oxidative/antioxidant mechanisms, genetic modulation, and cerebrovascular disease in children with sickle cell anemia

M Silva, P Faustino - Antioxidants, 2023 - mdpi.com
Sickle cell anemia (SCA) is a genetic disease caused by the homozygosity of the HBB: c.
20A> T mutation, which results in the production of hemoglobin S (HbS). In hypoxic …

Vascular instability and neurological morbidity in sickle cell disease: an integrative framework

H Stotesbury, JM Kawadler, PW Hales… - Frontiers in …, 2019 - frontiersin.org
It is well-established that patients with sickle cell disease (SCD) are at substantial risk of
neurological complications, including overt and silent stroke, microstructural injury, and …

Cerebrovascular manifestations in hematological diseases: an update

JM Ferro, J Infante - Journal of Neurology, 2021 - Springer
Patients with hematological diseases often experience cerebrovascular complications
including ischemic stroke, intracerebral and subarachnoid hemorrhage, microbleeds …

Silent cerebral infarcts in patients with sickle cell disease: a systematic review and meta-analysis

ME Houwing, RL Grohssteiner, MHG Dremmen, F Atiq… - BMC medicine, 2020 - Springer
Background and purpose Silent cerebral infarcts (SCIs) are the most common neurological
complication in children and adults with sickle cell disease (SCD). In this systematic review …

Hydroxyurea reduces cerebral metabolic stress in patients with sickle cell anemia

ME Fields, KP Guilliams, D Ragan… - Blood, The Journal …, 2019 - ashpublications.org
Chronic transfusion therapy (CTT) prevents stroke in selected patients with sickle cell
anemia (SCA). We have shown that CTT mitigates signatures of cerebral metabolic stress …